EWSR1-NFATC2 and FUS-NFATC2 Gene Fusion-Associated Mesenchymal Tumors: Clinicopathologic Correlation and Literature Review
Joint Authors
Exner, Ulrich G.
Bode-Lesniewska, Beata
Fuchs, Bruno
Fritz, Christine
Wagner, Ulrich
Source
Issue
Vol. 2019, Issue 2019 (31 Dec. 2019), pp.1-12, 12 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2019-03-26
Country of Publication
Egypt
No. of Pages
12
Main Subjects
Abstract EN
The spectrum of mesenchymal tumors associated with rearrangements of the EWSR1 gene has been growing in recent years due to progress in molecular detection techniques.
Originally identified as the gene involved in the pathogenesis of Ewing sarcoma, the EWSR1 gene is now known to be rearranged in diverse clinical and histopathological entities.
The NFATC2 gene is one of the many translocation partners of EWSR1 in gene fusions in a morphologically typical, albeit rare, subgroup of mesenchymal tumors.
Little is known about the clinical characteristics of tumors containing NFATC2 gene rearrangements since most of the few reports published describe molecular rather than clinical aspects.
In the current study, we report three patients with tumors carrying the EWSR1-NFATC2 gene translocation, including one rare primary tumor of soft tissues.
Another patient with a benign-appearing bone tumor with a unique FUS-NFATC2 gene translocation is described.
In various mesenchymal tumors (e.g., myxoid/round cell liposarcoma, low-grade fibromyxoid sarcoma, or angiomatoid fibrous histiocytoma), the FUS gene, as a member of the TET family, may be alternatively rearranged instead of the EWSR1 gene without any noticeable influence on the microscopical appearance or clinical outcome.
This fact seems not to apply to mesenchymal tumors with the involvement of the NFATC2 gene because both in our experience and according to the extensive literature review, they have different properties on the morphological and molecular level.
Both ESWSR1-NFATC2 and FUS-NFATC2 fusion-carrying tumors do not show microscopical or clinical features of Ewing sarcoma.
American Psychological Association (APA)
Bode-Lesniewska, Beata& Fritz, Christine& Exner, Ulrich G.& Wagner, Ulrich& Fuchs, Bruno. 2019. EWSR1-NFATC2 and FUS-NFATC2 Gene Fusion-Associated Mesenchymal Tumors: Clinicopathologic Correlation and Literature Review. Complexity،Vol. 2019, no. 2019, pp.1-12.
https://search.emarefa.net/detail/BIM-1207752
Modern Language Association (MLA)
Bode-Lesniewska, Beata…[et al.]. EWSR1-NFATC2 and FUS-NFATC2 Gene Fusion-Associated Mesenchymal Tumors: Clinicopathologic Correlation and Literature Review. Complexity No. 2019 (2019), pp.1-12.
https://search.emarefa.net/detail/BIM-1207752
American Medical Association (AMA)
Bode-Lesniewska, Beata& Fritz, Christine& Exner, Ulrich G.& Wagner, Ulrich& Fuchs, Bruno. EWSR1-NFATC2 and FUS-NFATC2 Gene Fusion-Associated Mesenchymal Tumors: Clinicopathologic Correlation and Literature Review. Complexity. 2019. Vol. 2019, no. 2019, pp.1-12.
https://search.emarefa.net/detail/BIM-1207752
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1207752